Santiesteban-Freixas R, Rodríguez-Hernández M, Hirano M, Francisco-Plasencia M, Mendoza-Santiesteban C, Carrero-Salgado M, Méndez-Larramendi I, Serrano-Verdura C, Luis-González S
Servicio de Neuroftalmología, Instituto de Neurología y Neurocirugía (INN), La Habana, Cuba.
Rev Neurol. 1999;29(4):289-96.
This century, the greatest epidemic affecting the nervous system was notified in Cuba seven years ago. At the present time the epidemic continues although to a lesser extent. The clinical findings of the illness were mainly bilateral optic neuropathy sometimes accompanied by other symptoms and peripheral neuropathy. The similarity of the optic form of the illness with Leber's hereditary optic neuropathy, and their common risk factors, were obvious from the beginning.
Statistics from the national reference department of neuro-ophthalmology of the Instituto de Neurologia de Cuba were reviewed. From these it was evident that the number of cases of Leber's hereditary optic neuropathy had increased in recent years, coinciding with the period of epidemic and endemic Cuban neuropathy. Many of these patients had previously been diagnosed as having epidemic optic neuropathy.
We describe the characteristics of a group of these patients and discuss the differences and possible relationship between the two conditions. In the case of epidemic optic neuropathy, there is strikingly simultaneous loss of vision, less visual changes with much smaller cecocentral scotomas, loss of ganglion fibres of the retina around the papillomacular bundle, a good response to multivitamin treatment, and increased frequency of association with peripheral sensory neuropathy.
This analysis reinforces the hypothesis that many patients with Leber's hereditary optic neuropathy, which started at the time of the epidemic, were incorrectly classified as suffering from this, and also perhaps their condition worsened due to the toxic nutritional features common to both conditions.
本世纪,七年前古巴通报了影响神经系统的最严重流行病。目前,该流行病仍在持续,尽管程度有所减轻。该疾病的临床症状主要为双侧视神经病变,有时伴有其他症状和周围神经病变。从一开始,该疾病的视神经病变形式与Leber遗传性视神经病变的相似性及其共同的危险因素就很明显。
回顾了古巴神经学研究所国家神经眼科参考部门的统计数据。由此可见,近年来Leber遗传性视神经病变的病例数有所增加,这与古巴流行性和地方性神经病变的时期相吻合。这些患者中有许多人之前被诊断为患有流行性视神经病变。
我们描述了一组这些患者的特征,并讨论了这两种疾病之间的差异和可能的关系。对于流行性视神经病变,视力丧失明显同时发生,视力变化较小,中心暗点较小,乳头黄斑束周围视网膜神经节纤维丧失,对多种维生素治疗反应良好,与周围感觉神经病变的关联频率增加。
该分析强化了这样一种假设,即许多在流行病时期开始发病的Leber遗传性视神经病变患者被错误地归类为此病,而且他们的病情可能由于这两种疾病共有的有毒营养特征而恶化。